Behcet disease
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Clinic
- BD is a type of inflammatory disorder which affects multiple parts of the body include painful sores on the mucous membranes of mouth and other parts of the body, inflammation of parts of the eye, and arthritis.
- Less commonly there may be inflammation of the brain or spinal cord, blood clots, aneurysms, or blindness.
Signs / Symptoms
Skin and mucosa
- Mouth painful ulcerations (Aphthous ulcers or Non-scarring oral lesions.(Similar to those found in IBD)
- Painful genital ulcerations around anus, vulva, or scrotum and cause scarring (75 %)
- Erythema nodosum,
- Cutaneous pustular vasculitis
- Pyoderma gangrenosum.
Eyes
- Anterior uveitis (Painful eyes, Conjuctival redness, Hypopyon and Decreased visual acuity)
- Posterior uveitis presents with painless decreased visual acuity and visual field floaters.
- Retinal vasculitis: (Painless decrease of vision with the possibility of floaters or visual field defects)
- Optic neuritis which progress to optic atrophy and visual loss
- Acute optic neuropathy (specifically anterior ischemic optic neuropathy). Optic nerve atrophy has been identified as the most common cause of visual impairment.
- Papilledema as a result of dural sinus thrombosis and atrophy resulting from retinal disease, have been characterized as secondary causes of optic nerve atrophy in Behçet's disease.
- Signs and symptoms of acute optic neuropathy include
- Painless loss of vision which may affect either one or both eyes,
- Reduced visual acuity
- Reduced color vision
- Relative afferent pupillary defect
- Central scotoma
- Swollen optic disc
- Macular edema
- Retrobulbar pain
- Episcleritis may occur, which causes eye redness and mild pain, without a significant impact on vision.
Bowels
- GI manifestations: Abdominal pain, Nausea, and Diarrhea with or without blood, and they often involve the ileocecal valve.
- Abdominal tenderness, Bloating, and General abdominal discomfort.
- Mild this can resemble IBS; more severe cases bear similarities to IBD.
Lungs
- Lung involvement is typically in the form of hemoptysis, Pleuritis, cough, or fever, and in severe cases can be life-threatening if the outlet pulmonary artery develops an aneurysm which ruptures causing severe vascular collapse and death from bleeding in the lungs.
- Nodules, consolidations, cavities and ground glass lesions are common in patients with pulmonary involvement.
- Pulmonary artery thrombosis may occur.
- Arthritis is seen in up to half of people, and is usually a non-erosive poly or oligoarthritis primarily of the large joints of the lower extremities.
Brain
- Chronic meningoencephalitis.
- Lesions of brainstem, basal ganglia and deep hemispheric white matter and may resemble those of MS.
- Brainstem atrophy is seen in chronic cases.
- Aseptic meningitis to
- Vascular thrombosis such as dural sinus thrombosis and organic brain syndrome manifesting with confusion, seizures, and memory loss.
- Sensorineural deafness
Heart
- Pericarditis
- Chronic aortic regurgitation due to aortic root disease may also be seen. Although infrequent, myocardial infarction (heart attack) with angiographically identified acute coronary artery thrombosis has been reported, including one case with a pathologically demonstrable lesion due to arteritis found at autopsy.
Blood vessels
- Blood vessel problems are observed in 7–29% of people with arterial lesions representing 15% of vascular lesions. Arterial lesions pose a greater risk. Most common arterial lesions are occlusions or stenosis and aneurysms or pseudoaneurysms.
Cause
- Streptococci (including S. sanguinis and S. pyogenes)
- Mycobacterium tuberculosis